Книга_4440_Библиотеки_им_академика_М_И_Перельмана

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Table 3.2 Different types of ear discharge with D/D

Types of discharge Denition Differential diagnosis Mucoid Thick discharge produced by

mucinous gland of middle ear cleft. It is characterized like forming rope

while cleaning Serous/watery It is watery CSF rhinorrhoea, ASOM Purulent Pus CSOM, furunculosis Mucopurulent Pus mixed with mucoid discharge CSOM (mucosal type) Serosanguinous/blood mixed Discharge mixed with blood ASOM, granulation, cholesteatoma Blood Bleeding from ear Trauma, haemangioma

Table 3.3 Different amounts of ear discharge with D/D

Amount Denition D/D Scanty Less in amount only seen on ngertip of bud while cleaning CSOM (cholesteatoma) Moderate Present in EAC, does not come out CSOM, otomycosis Copious Large in amount, spoil the pillow in night or in supine position ASOM, CSOM (mucosal),

3 History andExamination ofEar

CSOM (mucosal or tubotympanic type)

CSF otorrhoea

• Amount of discharge—Ear discharge can be copious or scanty in amount (Table3.3).

• Odour of discharge

– Foul smelling (unpleasant

odour)—cholesteatoma, anaerobic infection.

– Non-foul smelling—CSOM

(mucosal type), ASOM, otomycosis, furunculosis, wax, CSF otorrhoea.

• Frequency of discharge

– Continuous—trauma, bleeding

disorders, ASOM, CSF otorrhoea, wax.

– Episodic/intermittent—CSOM

(mucosal and squamous type), allergic, tubercular.

• Associated symptoms—Various symptoms may be associated with ear discharge like pain, fever, URI, nasal obstruction, vertigo, headache, neck swelling, etc.

– Earache

Earache followed by discharge—ASOM. Earache following discharge/ discharge followed by ear-

ache—otomycosis, acute exacerbation of CSOM. Earache persists with discharge— otomycosis, furunculosis.

– Fever

High grade fever with headache with discharge—CSOM/ASOM with meningitis, CSOM with subdural abscess, extradural abscess, brain abscess. High grade fever with chills and rigour with discharge—lateral

sinus thrombophlebitis. – Diplopia—petrositis – Pain

Retroorbital pain—CSOM with

petrositis.

Earache—furunculosis, otomy-

cosis.

Mastoid area—mastoiditis,

mastoid abscess. – Vertigo with discharge—CSOM

with lateral SSC stula.

– Swelling

Postaural swelling—postaural

abscess, mastoid abscess.

Neck swelling—Bezold’s

abscess, Citelli’s abscess.

3.1 Symptoms/History/Chief Complaints ofEar Diseases

Gradenigo’s syndrome (ear discharge+retroorbital pain+diplopia)

(b) Hearing loss—This is another common

symptom of ear disease. There are three types of hearing loss, conductive hearing loss (COHL), mixed hearing loss (MHL) and sensorineural hearing loss (SNHL) with which patient presents with different symptoms.

• Types of hearing loss—There are three types of hearing loss: conductive, sensorineural and mixed.

– Sensorineural hearing loss—

Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ (cochlea and associated structures) or the ves-

tibulocochlear nerve (cranial nerve

VIII). This is divided into sensory hearing loss, which is due to cochlear pathology; and neural hearing loss, which is due to pathology in cochlear nerve and pathway.

– Conductive hearing loss—The

conductive hearing loss developed when sounds cannot get through the outer and middle ear. It may be hard to hear soft sounds, and louder sounds may be mufed. Medicine or surgery can often x this type of hearing loss.

– Mixed hearing loss—MHL is a con-

dition when both the components, conductive hearing loss and sensorineural hearing loss, are present. It is caused by the combination of conductive damage in the outer or the middle ear and sensorineural damage in the inner ear or the auditory nerve. A person with mixed hearing loss will suffer from either permanent hearing loss or the temporary hearing loss. Mixed hearing loss can affect either one ear or both ears.

• Causes of hearing loss (Table 3.4)

• Presentation of hearing loss – Sensorineural hearing loss—The

main symptoms with which patient of SNHL may present are

Trouble following group conversations. Mufed conversation sounds. Loss of acuity in distinguishing foreground voices against noisy backgrounds. Difculty hearing high-pitched sounds. Difculty in understanding telephonic conversation. Some kinds of sounds seeming excessively loud or shrill. Difculty understanding some parts of speech. Loss of directionality of sound (especially with high-frequency sounds) (fricatives and

sibilants).

Table 3.4 Causes of hearing loss

COHL SNHL Mixed Wax

Furunculosis Foreign body EAC atresia and stenosis ASOM, AOM Tympanosclerosis suppurative otitis media (SOM) CSOM Cholesteatoma Ossicular xation Ossicular erosion Otosclerosis

Ageing Noise-induced Drug side effect Auditory nerve tumour, acoustic trauma Barotrauma Presbyacusis Cochlear agenesis Labyrinthitis

Genetic infection, chronic otitis media (COM), labyrinthitis) Head trauma (involve inner ear or nerve) Otosclerosis Ototoxic drugs Allergy Abnormality of the outer ear, ear canal or middle ear structure

Table 3.5 Causes of U/L and B/L hearing loss

Unilateral causes Bilateral causes COHL—CSOM, ASOM, AOM, SOM wax,

furunculosis, otosclerosis, retracted TM, adhesive otitis media

SNHL—idiopathic, temporal bone fracture, rupture of oval or round window membrane, perilymph stula Meniere’s disease, vestibular schwannoma

MHL—cochlear otosclerosis MHL—otosclerosis

COHL—CSOM, SOM, adhesive otitis media, retracted TM

SNHL—presbyacusis, drug-induced, noise-induced, autoimmune diseases

3 History andExamination ofEar

Perception that people mumble when speaking. Difculty understanding speech. Associated symptoms are vertigo and SNHL. Tinnitus Vertigo Ear discharge H/o drugs, trauma, noise exposure, DM, HT, fever, ototoxic drugs, barotrauma, acoustic trauma

– Conductive hearing loss—Patient

with COHL is presented in a different way:

Patients hear better in noisy environment (Paracusis Willisii). Difculty in hearing speech. A sense that own voice sounds different. Easier to hear out of one ear than the other. The sensation of pain in one or both ears. A sensation of pressure in one or both ears. Associated h/o ear discharge, physical trauma, earache, heaviness in ear, URI, nasal obstruction, acoustic trauma, barotrauma.

– Mixed hearing loss—MHL is pre-

sented in various ways:

Loss of clarity while listening to sounds.

Inability to hear at a normal hearing level. A consistent ringing or buzzing in the ears. Partial or full loss of hearing. A sensation of pressure in one or both ears. Difculty in following up the conversations.

• Side of hearing loss—Hearing loss

may affect one ear and/or both ears simultaneously or at different times. There are various causes (Table3.5).

• Time of onset—Congenital or

acquired

– Congenital hearing loss—It is

due to maldevelopment of cochlea and presented either alone or in association with other deformities. It has been classied into two subgroups. The rst is syndromic congenital hearing loss, and second one is non-syndromic congenital hearing loss.

Syndromic congenital hearing loss—Almost 30% of patients

of congenital hearing loss belong to this group. Depending on time of onset, syndromic hearing loss can become apparent at any time from infancy to old age (Table3.6).

Differential diagnosis of syndromic congenital hearing loss Non-syndromic congenital hearing loss (NSSHL)—Non-

3.1 Symptoms/History/Chief Complaints ofEar Diseases

Table 3.6 Differential diagnosis of syndromic hearing loss

Clinical features Syndrome (autosomal dominant) D/D

U/L or B/L SNHL, pigmentary changes—like greying hair, white forelock, heterochromia iridis, partial albinism, facial features—fused eyebrows, high nasal bridge, hypoplastic alae nasi

Hearing loss is COHL or MHL or SNHL Others are preauricular sinus, branchial cyst or sinus, renal problem+Mondini’s dysplasia

Hearing loss (SNHL, COHL or MHL) may be progressive Micrognathia (small jaw) with cleft palate (Pierre Robin sequence)

Coloboma, choanal atresia, cranial nerve problems and unusually shaped ears. Hearing loss may be conductive, sensorineural or mixed (mild to profound)

Hearing loss (conductive), malar hypoplasia, microtia/anotia, stenosis/ atresia of EAC, ear tag, etc.

Progressive hearing loss (SNHL), B/L, acoustic neuroma by 30years of age

SN hearing loss (profound and congenital), vestibular dysfunction (vertigo), no benet with hearing aid

Moderate to severe, downward sloping, congenital SNHL, no vestibular dysfunction

Progressive, sensorineural hearing loss, later onset Usher’s syndrome type 3 Hearing loss is sensorineural and may be progressive. Inner ear

malformations (Mondini’s malformation) may be present Balanced dysfunction is present Enlarged thyroid gland (goitre)

Bilateral, profound sensorineural hearing loss is present at birth Abnormal heart rhythm (long Q-T) Syncopal episode and possible sudden death

Hearing loss is progressive and sensorineural kidney problems (nephritis), near-sightedness (myopia)

Hearing loss in males is mixed and may be progressive X-linked congenital stapes xation with

Hearing loss is sensorineural and often found in the high frequencies Diabetes

Hearing loss is bilateral and sensorineural Unsteady gait (ataxia), short stature, delayed puberty, progressive paralysis of the eye muscles (ophthalmoplegia) and progressive blindness (retinopathy)

Hearing loss in >75% of cases Skin rashes, alopecia, seizure, ataxia

Waardenburg syndrome (most common)

Branchio-oto-renal syndrome

Stickler syndrome

CHARGE syndrome

Treacher Collins syndrome

Neurobromatosis type II (NFII)

Autosomal recessive syndrome

Usher syndrome type 1

Usher’s syndrome type 2

Pendred syndrome

Jervell and Lange-Nielsen syndrome

X-linked syndrome

Alport’s syndrome

perilymph gusher

Mitochondrial

Maternally Inherited Diabetes and Deafness (MIDD)

Kearns-Sayre syndrome (KSS)

Biotinidase deciency

syndromic congenital hearing loss is a partial or total loss of hearing that is not associated with other signs and symptoms. Non-syndromic congenital hearing loss is restricted to the inner ear congenital deformi-

ties. The characteristics of nonsyndromic hearing loss vary among the different types. Nonsyndromic congenital hearing loss (NSSHL) can further be categorized by its mode of inheritance into two. The rst

Table 3.7 Differential diagnosis of non-syndromic hearing loss

History of clinical features Name of deformities or D/D Profound hearing loss with LAMM

syndrome SNHL—The hearing loss can deteriorate

over time either gradually or in a stepwise fashion or may be a profound HL since birth

Profound SNHL Scheibe dysplasia (cochleosaccular dysplasia or pars inferior dysplasia

High-frequency hearing loss Alexander aplasia (basal turn of membranous cochlea) limited

SNHL progressive Familial progressive sensorineural deafness

Michel aplasia (complete labyrinthine aplasia)

Mondini’s dysplasia—It is a cochlear anomaly (incomplete partition) rst described by Mondini in 1791

or limited membranous labyrinthine dysplasia)—It is rst described by Scheibe in humans. It is a common cause of profound SNHL

membranous labyrinthine dysplasia

Metabolic disorders, congenital ossicular xation

3 History andExamination ofEar

one is autosomal dominant (DFNA), which accounts 20%

of NSSHL. This is postlingual and delayed in onset. The second one is autosomal recessive (DFNB), which accounts 80% of inherited NSSHL (DFNB), in which hearing loss is generally congenital. It is usually prelingual, non-progressive and severe to profound (Tables 3.6 and 3.7).

which means they are associated with a permanent loss of hearing caused by damage to structures in the inner ear. Less commonly, non-syndromic hearing loss is described as conductive, meaning it results from changes in the middle ear. Some forms of non-syndromic hearing loss, particularly a type called DFNX2, involve changes in both the inner ear and the middle ear. This combination is called mixed hearing loss.

Characteristic Features of Non-syndromic Congenital Hearing Loss

Hearing loss can affect one ear (unilateral) or both ears (bilateral).

Degrees of hearing loss range from mild (difculty understanding soft speech) to profound (inability to hear even very loud noises).

The hearing loss can be stable, or it may be progressive, becoming more severe as a person gets older. Particular types of nonsyndromic hearing loss show distinctive patterns of hearing loss. For example, the loss may be more pronounced at high, middle or low tones.

The most forms of non-syndromic hearing loss are described as sensorineural,

History to Be Ruled Out

History of (h/o) intrauterine infection Family history of congenital hearing loss Low birth weight (<1500g) Hyperbilirubinaemia Prolonged neonatal intensive care unit stay or prolonged mechanical ventilation Concurrent craniofacial anomalies Exposure to ototoxic medications <5 Apgar score at 1min, <7 Apgar score at 5min Bacterial meningitis

Differential diagnosis of nonsyndromic congenital hearing loss

3.1 Symptoms/History/Chief Complaints ofEar Diseases

• Acquired hearing loss—Hearing loss presents after birth at any time in one’s life as a result of illness or injury or genetic factors. Depending on time onset of hearing loss, this has divided into three groups (Table3.8).

– Prelingual acquired hearing

loss—Prelingually, deaf child is one either who is born deaf or who lost his or her hearing early in childhood, before acquiring language. Prelingual hearing loss occurs before a child develops speech and language, and is usually associated with severe or profound hearing loss. This type of hearing issue appears in newborn children and infants up to the age of three, as their language skills have not yet fully developed.

– Postlingual acquired hearing

loss—The postlingual deafness is a deafness, which develops after the acquisition of speech and language, usually after the age of 6.

Typically, hearing loss is gradual, and often detected by family and friends of the people so affected long before the patients themselves will acknowledge the disability.

– Perilingual hearing loss—The

perilingual hearing loss develops during the development of hearing and speech.

– Causes of acquired hearing loss

• Duration of hearing loss – Acute hearing loss (<3months)—

If total duration of hearing loss is less than 3months, it is used to be considered an emergency (Table3.9).

– Presentation of acute hearing

loss

– Chronic hearing loss

(>3 months)—if the duration of symptoms is more than 3 months (Table3.10).

– Presentation of chronic hearing

loss

Table 3.8 Causes of acquired hearing loss

Prelingual acquired hearing loss Perilingual acquired hearing loss Postlingual acquired hearing loss Symptoms—No speech if profound

hearing loss; 5in every 1000 babies suffer some degree of hearing loss

Positive history of maternal infection with rubella, measles, premature delivery, ear infection, meningitis

Table 3.9 Presentation and causes of acute hearing loss

Symptoms/presentations Causes Occurrence of acute hearing loss usually only in one ear

Sudden occurrence of acute hearing loss within 24h No ear pain Tinnitus or other ear sounds Pressure sensation in the ear Dizziness in severe cases

Symptoms—Some speech develops Symptoms—Speech development

present even in profound hearing loss

Positive history of meningitis, trauma

Viral infections or reactivations (herpes) Arteriosclerosis Metabolic diseases such as diabetes or high cholesterol levels Rupture of the round or oval windows Cervical spine disorders (whiplash) Elevated aggregation of thrombosis Blockage (thrombosis) of the inner ear vessel Autoimmune disorders Acute otitis media

Diseases/causes—drug- induced, presbycusis (age related), noiseinduced, otitis media Diseases/causes—meningitis, hypertension, DM

Table 3.10 Presentation and causes of chronic hearing loss

Symptoms/presentation D/D Hearing loss in old age Presbycusis (SNHL) Hearing loss since birth (deafmute) Hereditary (SNHL) Episodic/uctuating Meniere’s disease (SNHL) Exposure to loud noise Noise-induced SNHL Infective, h/o ear discharge CSOM (COHL or MHL) Infective, no h/o ear discharge SOM (COHL) Blocking sensation in ear Retracted TM (COHL) H/o drug intake like aminoglycoside,

chemotherapeutic agent, non steroidal anti inammatory drug (NSAIDs), etc.

Progressive hearing loss U/L or B/L Otosclerosis (COHL or MHL)

Table 3.11 Causes of gradual onset hearing loss

COHL SNHL MHL U/L B/L U/L B/L U/L B/L CSOM

SOM Adhesive otitis media Wax Otomycosis

B/L SOM Retracted TM B/L CSOM

Acoustic neuroma

Drug-induced (SNHL)

Acoustic neuroma Presbyacusis Drug- induced, noise- induced

3 History andExamination ofEar

Otosclerosis COM with labyrinthitis

Otosclerosis

• Mode of onset—Hearing loss can develop gradually or suddenly.

– Gradual onset—The gradual

onset hearing loss occurs over time. It can affect either one ear (CSOM) or both ears (otosclerosis, presbyacusis, noise-induced, druginduced) (Table3.11).

– Causes of gradual onset hearing

loss

– Sudden onset—sudden onset

hearing loss (SOHL) is a subjective symptom in one or both ears. It causes great concern for the patient, especially when there is a 30dB or greater hearing loss over less than 72h/3days in 3 frequencies. It is of rapid onset, typically occurring within a few days. SOHL may be conductive or SNHL or

mixed hearing loss. Hearing loss can range from mild hearing impairment to a total loss of hearing, and it may be temporary or permanent (Table3.12).

– Causes of sudden onset hearing

loss

• Progression of hearing loss – Progressive hearing loss—Hearing

loss increases in severity since its onset (Table3.13).

– Causes of progressive hearing

loss

– Non-progressive hearing loss—If

the hearing loss does not change over time, it is termed as ‘nonprogressive’ or stable, static or not deteriorating (Table3.14).

– Causes of non-progressive hear-

ing loss

3.1 Symptoms/History/Chief Complaints ofEar Diseases

Table 3.12 Sudden onset hearing loss

COHL U/L SNHL U/L MHL U/L

Foreign body Wax Otitis externa (OE) Head injury or trauma Haemotympanum

COHL B/L SNHL B/L MHL B/L

Barotrauma, ASOM Autoimmune system diseases such

Table 3.13 Causes of progressive hearing loss

COHL U/L SNHL U/L MHL U/L

Otosclerosis CSOM, SOM, retracted TM

COHL B/L SNHL B/L MHL B/L

CSOM, otosclerosis, retracted TM Presbycusis, meningitis Otosclerosis

Table 3.14 Causes of non-progressive hearing loss

COHL U/L SNHL U/L MHL U/L

Ossicular discontinuity, CSOM with permanent perforation, atelectatic TM

COHL B/L SNHL B/L MHL B/L

Adhesive otitis media, atelectatic TMPostmeningitis Genetic disorder

Idiopathic, infective, noise-induced, drug-induced, ototoxic medication, trauma (temporal bone fracture), perilymphatic stula

as Cogan’s syndrome, Meniere’s

disease, Lyme disease, snake bite.

Neurological conditions, such as

multiple sclerosis, acoustic trauma

Presbycusis, noise-induced HL, drug-induced HL

Cochlear dysplasia Postmeningitis, postlabyrinthitis

Otosclerosis, head trauma, infection

Otosclerosis, genetic disorder

Otosclerosis

Genetic disorder

Leading Question to Be Asked for Evaluation of Hearing Loss

When did patient’s hearing loss started? How did hearing loss progress either suddenly or slowly? Does hearing loss involve one ear or both ears? Is there any history of ear discharge, dizziness, pain, heaviness and tinnitus? Any family history of hearing? Profession of patient and noise level at profession place? Any history of ear infection, trauma, stroke, diabetes, stroke and meningitis? Any history of medication for Diabetes mallitus (DM), TB, carcinoma and other ototoxic drugs?

• Associated symptoms and history – Associated symptoms like ear dis-

charge, vertigo, tinnitus, heaviness, pain.

– History of birth and pregnancy

information, medical history, development history, workplace environment, home environment.

– History of drug exposure—expo-

sure of ototoxic drugs, either systemically or locally like aminoglycosides.

– History of trauma, acoustic trauma,

surgery.

– Family history of three generation

with hearing loss either syndromic or non-syndromic.

Table 3.15 Vertigo-like symptoms

Symptoms Denition Dizziness Sensation of perturbed/disturbed spatial orientation Disequilibrium Sensation of being off balance or unstable Oscillopsia Illusion of oscillation of visual environment Gait ataxia Imbalance of appearing drunk when ambulating. It may be cerebellar or sensory Syncope, presyncope Feeling of faintness or near- faintness, cardiovascular, autonomic and orthostatic

hypotension

Light headedness Sensation giddiness

3 History andExamination ofEar

– History of noise exposure—

Exposure to a single very loud noise such as a gunshot or bomb blast can cause noise-induced hearing loss, e.g. using headphones at high volume over time, or being in loud environments regularly, such as a loud workplace, sporting events, concerts, and using noisy machine. Noisy machines can also be a risk for noise-induced hearing loss.

unpleasant disturbance of spatial orientation or the illusionary perception of a movement (spinning and wobbling) of the body and/or of the surroundings.

Vertigo-like symptoms (Table 3.15) Classication/types of vertigo—The

vertigo has been classied according to rotation, stimulus involved, patient perception, site of lesion and periodicity of vertigo.

Induced—BPPV, cervical spondylosis,

hypermobile stapes syndrome, dehiscence of SSCC

Patient’s perception

Subjective (feel like moving or swaying)—

chronic subjective dizziness (BPPV)

Objective (feel like surroundings are mov-

ing)—Meniere’s disease

Depend on site of lesion

Peripheral (due to involvement of inner ear

and vestibular nerve)—BPPV, vestibular neuritis, labyrinthitis

Central—hallucinations of motion of their

surroundings, or a sensation of spinning, while remaining still, as a result of dysfunction of the vestibular structures in the central nervous system (CNS)— brainstem or cerebellar stroke, vestibular migraine

Periodicity of vertigo

Episodic vertigo—This is dened as brief

episodes of intermittent, recurrent vertigo lasting seconds to hours with asymptomatic period in between

Rotation

Rotatory—vestibular neuronitis, BPPV,

Meniere’s disease

Non-rotatory (imbalance)—drug intoxica-

tion, phobic vertigo

Stimulus involved

Spontaneous—acute spontaneous vertigo

(vestibular neuronitis, labyrinthitis, brainstem or cerebellar stroke)

Attacks—vestibular migraine, PBBV, otitic syphilis, Cogan’s disease, vasovagal attack, cervical spondylitis.

Constant vertigo—This is dened as con-

tinuous vertigo or dizziness does not relieve without medication. It can be caused by vestibular neuritis, central vertigo, vestibular neuronitis, stroke, drug intoxication.

3.1 Symptoms/History/Chief Complaints ofEar Diseases

Difference between peripheral and

central vertigo (Table 3.16)

• Presentation/chief complaints of vertigo

– In children—Children with ver-

tigo may be presented as sudden cry, drop on oor, cling to legs of adults, pallor, sweating, vomiting, lying face down on bed and reluctant to move.

– In adult—Adult with vertigo pres-

ents as a sensation of movement of the environment. Often, patients describe a ‘spinning’ sensation of either their body or their surroundings.

• Duration of vertigo—Vertigo has been classied into acute and chronic (Table3.17).

Table 3.16 Features or difference of peripheral and central vertigo

Features Peripheral Central Nystagmus Horizontal

Inhibited by xation of vision Fades after few days Fixed direction

Fatigability Imbalance Mild to moderate Severe Nausea, vomiting Severe Vary Audiological symptoms Common Rare Neurological symptoms Rare Common Latency Present Absent Site of lesion Vestibular system Central Onset Sudden Gradual Severity Intense Mild Pattern Paroxysmal Constant Laterality Unilateral Uni/bilateral

• Duration of episode of vertigo (Table

3.18)

• Severity—The vertigo has been classied into mild, moderate and severe (Table3.19).

• Mode of onset—The mode of onset of vertigo can be sudden or gradual (Table3.20).

• Time of onset—Vertigo presents at birth is congenital or develops later on is acquired (Table3.21).

• Progression—Vertigo can be progressive or non-progressive.

• Progressive—The severity of vertigo increases with the time termed as progressive. It is caused by Meniere’s disease, vestibular neuronitis, vestibular labyrinthitis, acoustic neuroma.

Rotatory, vertical Not inhibited by xation of vision Last for week or month Directional changing Not fatigable

Table 3.17 Causes of acute and chronic vertigo

Acute vertigo (<3months) Chronic vertigo (>3months) Acute idiopathic unilateral vestibulopathies (vestibular

neuronitis, acute labyrinthitis) Cerebellar stroke BPPV Migrainous vertigo Meniere’s disease Missed BPPV Cholesteatoma Vertebral or basilar artery insufciency Oscillopsia Wallenberg’s disease

Chronic labyrinthitis

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